Bleeding Disorders — MCQs
A 78 year old man who lives alone and prepares his own food is found to have ecchymotic patches over posterior aspect of his lower extremities. He has haemorrhagic areas around hair follicles, hair are fragmented and splinter haemorrhages are present in nail beds and several hematomas are present in the muscles of arms and legs. PT and CT are normal. This clinical syndrome is most likely due to
The most common bleeding manifestation of Hemophilia is
Which of the following is NOT a contraindication for spinal anaesthesia?
Adenoidectomy is contraindicated in:
A 45-year-old lady presents with normal PT and increased aPTT. About 2 years ago, she underwent cholecystectomy and did not have any bleeding episode. What is the next investigation for clinical diagnosis?
Which of the following statements are correct in respect of haemophilia? 1. It is hereditary bleeding disorder. 2. It has a higher incidence among Asians. 3. It affects females occasionally and mildly.
A 6-year-old child with abdominal pain and a rash is shown. Comment on the diagnosis?
A 5-year-old boy presents with petechial bleeding and bruising on his torso and limbs. He has no other signs or symptoms and does not appear ill. His mother reports a gastrointestinal infection several weeks prior to the onset of petechiae and bruising. Complete blood count reveals thrombocytopenia (<20 x 10^9/L), with other parameters within the expected range for his age. Prothrombin time, partial thromboplastin time, and metabolic panels are all within the reference range. What is the expected outcome of this blood disorder?
A 21-year-old male presents with anemia and mild hepatosplenomegaly. His hemoglobin is 5 gm/dL, and he has a history of a single blood transfusion to date. What is the most probable diagnosis?
What is the recommended treatment for Kostmann's syndrome?
Bleeding Disorders Indian Medical PG Practice Questions and MCQs
Question 1: A 78 year old man who lives alone and prepares his own food is found to have ecchymotic patches over posterior aspect of his lower extremities. He has haemorrhagic areas around hair follicles, hair are fragmented and splinter haemorrhages are present in nail beds and several hematomas are present in the muscles of arms and legs. PT and CT are normal. This clinical syndrome is most likely due to
- A. Vitamin A deficiency
- B. Vitamin C deficiency (Correct Answer)
- C. Vitamin K deficiency
- D. Pyridoxine deficiency
Explanation: ***Vitamin C deficiency*** - The constellation of **ecchymotic patches**, **perifollicular hemorrhages** with fragmented hairs, **splinter hemorrhages**, and **muscle hematomas** is classic for **scurvy**, caused by vitamin C deficiency [2]. - Vitamin C is essential for **collagen synthesis**, and its deficiency leads to weakened blood vessel walls and connective tissue, resulting in these bleeding manifestations [2]. *Vitamin A deficiency* - Primarily causes **ocular symptoms** such as **night blindness** and **xerophthalmia**, and can also lead to **hyperkeratosis follicularis** (bumpy skin) [1]. - It does not typically cause the diffuse ecchymoses, perifollicular hemorrhages, or muscle hematomas seen in this patient. *Vitamin K deficiency* - Leads to impaired synthesis of **coagulation factors II, VII, IX, and X**, resulting in prolonged **PT (prothrombin time)** and **aPTT (activated partial thromboplastin time)** [3]. - The patient's normal PT and CT strongly argue against vitamin K deficiency as the primary cause of bleeding [3]. *Pyridoxine deficiency* - Also known as **vitamin B6 deficiency**, it can cause **neurological symptoms** (neuropathy, seizures), **dermatitis**, and **sideroblastic anemia**. - It is not associated with the extensive bleeding and connective tissue manifestations described in this clinical scenario.
Question 2: The most common bleeding manifestation of Hemophilia is
- A. Hemarthrosis (Correct Answer)
- B. Hematuria
- C. Hemoptysis (coughing up blood)
- D. Hematomas (bleeding into soft tissues)
Explanation: ***Hemarthrosis*** - Characterized by **bleeding into joint spaces**, leading to pain, swelling, and reduced mobility, commonly seen in Hemophilia [1]. - Occurs due to deficiency of clotting factors, resulting in joint bleeding with minimal trauma [1]. *Hematuria* - Refers to the presence of **blood in urine**, which is often associated with **urinary tract conditions**, not primarily Hemophilia. - While it can occur in hemophilia patients, it is less characteristic of Hemophilia compared to hemarthrosis. *Hematomas* - These are localized collections of **blood outside of blood vessels**, typically resulting from blunt trauma or injury. - Although they can occur in Hemophilia as soft tissue hemorrhage [1], they are not as specific as hemarthrosis for bleeding manifestations. *Hemoptysis* - Defined as coughing up **blood**, which usually indicates pulmonary or vascular conditions rather than a bleeding disorder like Hemophilia. - It is not a common manifestation of Hemophilia, which primarily presents with joint and muscle bleeding [1].
Question 3: Which of the following is NOT a contraindication for spinal anaesthesia?
- A. Raised intracranial tension
- B. Bleeding disorder
- C. Hypertension (Correct Answer)
- D. Infection at injection site
Explanation: ***Hypertension*** - While **severe uncontrolled hypertension** may necessitate blood pressure stabilization before surgery, **mild to moderate hypertension** is not an absolute contraindication for spinal anesthesia. - In fact, spinal anesthesia can sometimes be beneficial in hypertensive patients due to its **vasodilatory effects**, which may help lower blood pressure. *Bleeding disorder* - A **bleeding disorder** (e.g., thrombocytopenia, coagulopathy) is a **major contraindication** due to the high risk of **epidural or spinal hematoma** formation. - A hematoma can lead to **spinal cord compression** and irreversible neurological damage. *Raised intracranial tension* - **Raised intracranial tension (ICT)** is a **strict contraindication** because the drop in cerebrospinal fluid (CSF) pressure during spinal anesthesia can worsen the pressure gradient across the foramen magnum. - This can precipitate **herniation of the brainstem** and lead to catastrophic neurological injury or death. *Infection at injection site* - The presence of an **infection at the injection site** is an absolute contraindication as it poses a significant risk of introducing bacteria into the **subarachnoid space**. - This can lead to serious complications such as **meningitis** or a **spinal abscess**.
Question 4: Adenoidectomy is contraindicated in:
- A. SOM
- B. CSOM
- C. Bleeding disorder (Correct Answer)
- D. None of the options
Explanation: ***Bleeding disorder*** - Adenoidectomy involves surgical removal of tissue, which carries a risk of **intraoperative and postoperative bleeding**. - In individuals with a **pre-existing bleeding disorder**, this risk is significantly elevated, potentially leading to serious complications. *SOM* - **Serous otitis media (SOM)**, or otitis media with effusion, is often caused by Eustachian tube dysfunction, which can be exacerbated by adenoid hypertrophy. - Adenoidectomy can actually be a **treatment for recurrent SOM**, as it can relieve obstruction of the Eustachian tube. *CSOM* - **Chronic suppurative otitis media (CSOM)** involves a persistent perforation of the tympanic membrane with chronic ear discharge. - While adenoid hypertrophy can contribute to Eustachian tube dysfunction and recurrent acute otitis media that might lead to CSOM, an adenoidectomy is **not directly contraindicated** for CSOM itself. *None of the options* - This option is incorrect because **bleeding disorder** is a clear contraindication for adenoidectomy due to the increased risk of hemorrhagic complications.
Question 5: A 45-year-old lady presents with normal PT and increased aPTT. About 2 years ago, she underwent cholecystectomy and did not have any bleeding episode. What is the next investigation for clinical diagnosis?
- A. Platelet aggregation test
- B. Ristocetin cofactor assay
- C. Factor VIII assay
- D. Dilute Russell viper venom assay (Correct Answer)
Explanation: ***Dilute Russell viper venom assay*** - A normal PT and an increased aPTT, along with a history of no bleeding during surgery, are highly suggestive of **antiphospholipid syndrome** (APS). [1] - The dilute Russell viper venom time (DRVVT) is a specific and sensitive test for detecting **lupus anticoagulant**, a key feature of APS. [1] *Platelet aggregation test* - This test evaluates platelet function and is used for diagnosing primary **platelet disorders** like von Willebrand disease or Bernard-Soulier syndrome. - It would not be the primary next step for an isolated prolonged aPTT with no bleeding history, as this pattern points to a **coagulation factor issue** or an anticoagulant. [1] *Ristocetin cofactor assay* - This assay is primarily used to diagnose **von Willebrand disease** by measuring the activity of von Willebrand factor (vWF). [2] - While von Willebrand disease can cause a prolonged aPTT, the absence of bleeding during a major surgery (cholecystectomy) makes a severe vWD diagnosis less likely, and APS is a more probable cause in this context. [3] *Factor VIII assay* - A Factor VIII deficiency (Hemophilia A) can cause a prolonged aPTT, but severe deficiencies typically lead to **significant bleeding episodes**, especially after surgery. [3] - The lack of bleeding during cholecystectomy makes a clinically significant Factor VIII deficiency less likely, and it wouldn't explain the normal PT. [1]
Question 6: Which of the following statements are correct in respect of haemophilia? 1. It is hereditary bleeding disorder. 2. It has a higher incidence among Asians. 3. It affects females occasionally and mildly.
- A. 1 and 3 only (Correct Answer)
- B. 2 and 3 only
- C. 1 and 2 only
- D. 1, 2 and 3
Explanation: ***1 and 3 only*** - Haemophilia is a well-known **hereditary bleeding disorder**, primarily affecting males, caused by a deficiency in certain clotting factors [1]. - While haemophilia mainly affects males, females can be carriers and, in rare instances, experience mild to moderate symptoms, especially due to **extreme X-inactivation**. *2 and 3 only* - The statement that it has a higher incidence among Asians is **incorrect**; haemophilia incidence is generally consistent across ethnic groups, approximately 1 in 5,000 live male births worldwide [2]. - While females can be affected, their involvement is typically mild and occasional, making statement 3 correct. *1 and 2 only* - Haemophilia is indeed a **hereditary bleeding disorder** (statement 1 is correct) [1]. - However, the claim of higher incidence among Asians is **unsupported** by epidemiological data, rendering statement 2 incorrect. *1, 2 and 3* - While haemophilia is a **hereditary bleeding disorder** and can affect females, the assertion of a higher incidence among Asians is **false** [2]. - Its global incidence rates do not show a significant racial or ethnic predilection.
Question 7: A 6-year-old child with abdominal pain and a rash is shown. Comment on the diagnosis?
- A. Kawasaki
- B. Varicella
- C. Meningococcemia
- D. Henoch-Schonlein purpura (Correct Answer)
Explanation: ***Henoch Schonlein purpura*** - This diagnosis is strongly suggested by the child's age (6 years old), presentation of abdominal pain, and the characteristic **palpable purpuric rash**, particularly on the lower extremities, as seen in the image. - **Henoch-Schönlein purpura (HSP)**, now known as IgA vasculitis, is a systemic small-vessel vasculitis predominantly affecting children, characterized by the classic triad of palpable purpura, arthritis/arthralgia, and abdominal pain. *Kawasaki* - **Kawasaki disease** primarily affects children under 5 years of age and presents with persistent fever, conjunctivitis, oral mucosal changes (strawberry tongue), cervical lymphadenopathy, and a polymorphous rash. Abdominal pain is less common as a primary feature. - The rash in Kawasaki disease is typically not purpuric but can be maculopapular or scarlatiniform, and does not show the characteristic distribution seen in the image. *Varicella* - **Varicella (chickenpox)** is characterized by a pruritic vesicular rash that progresses from macules to papules to vesicles and then crusts, usually starting on the trunk and spreading centrifugally. This is distinctly different from the purpuric rash shown. - While it can cause abdominal pain, the skin lesions are the key differentiator, and the image does not depict vesicular lesions. *Meningococcemia* - **Meningococcemia** is a severe bacterial infection often presenting with petechial or purpuric rash, fever, and signs of sepsis. However, the rash in meningococcemia rapidly progresses to large ecchymoses and is often associated with signs of critical illness (e.g., hypotension, altered mental status). - While purpura is present, the widespread, relatively uniform appearance of the rash, combined with abdominal pain in a 6-year-old, points away from the fulminant course typical of meningococcemia towards a vasculitis like HSP.
Question 8: A 5-year-old boy presents with petechial bleeding and bruising on his torso and limbs. He has no other signs or symptoms and does not appear ill. His mother reports a gastrointestinal infection several weeks prior to the onset of petechiae and bruising. Complete blood count reveals thrombocytopenia (<20 x 10^9/L), with other parameters within the expected range for his age. Prothrombin time, partial thromboplastin time, and metabolic panels are all within the reference range. What is the expected outcome of this blood disorder?
- A. Complete resolution is expected. (Correct Answer)
- B. Survival rate is up to 70% depending on risk stratification.
- C. Lifelong disease dependent on factor VIII substitution.
- D. Lifelong disease dependent on factor IX substitution.
Explanation: ### Explanation The clinical presentation describes a classic case of **Immune Thrombocytopenic Purpura (ITP)**, the most common cause of isolated thrombocytopenia in children. **1. Why Option A is Correct:** In children, ITP typically follows a viral infection (respiratory or gastrointestinal) after a 1–4 week latent period. It is characterized by the sudden onset of petechiae and bruising in an otherwise healthy-appearing child. The hallmark is **isolated thrombocytopenia** (Platelets <100 x 10⁹/L) with normal PT, PTT, and hemoglobin. The prognosis is excellent; approximately **70–80% of children achieve complete spontaneous resolution** within 6 months, regardless of therapy. **2. Why the Other Options are Incorrect:** * **Option B:** This refers to the survival rates of pediatric malignancies like Acute Lymphoblastic Leukemia (ALL). While ALL presents with bruising, it typically involves "sick" symptoms (fever, bone pain), hepatosplenomegaly, and abnormalities in other cell lines (anemia/leukocytosis). * **Options C & D:** These describe **Hemophilia A (Factor VIII)** and **Hemophilia B (Factor IX)**. Hemophilias are coagulation factor deficiencies that present with deep tissue bleeds (hemarthrosis/hematomas) and a **prolonged aPTT**, rather than petechiae and isolated thrombocytopenia. **3. NEET-PG High-Yield Pearls:** * **Pathophysiology:** Anti-platelet antibodies (IgG) directed against GP IIb/IIIa or GP Ib/IX. * **Bone Marrow:** Not routinely required but would show **increased megakaryocytes** (compensatory). * **Management:** Observation is preferred if bleeding is minimal (dry purpura). If treatment is needed (wet purpura/active bleeding), **IVIG** or **Corticosteroids** are first-line. * **Chronic ITP:** Defined as thrombocytopenia persisting >12 months (occurs in ~20% of cases).
Question 9: A 21-year-old male presents with anemia and mild hepatosplenomegaly. His hemoglobin is 5 gm/dL, and he has a history of a single blood transfusion to date. What is the most probable diagnosis?
- A. Thalassemia major
- B. Thalassemia minor
- C. Thalassemia intermedia
- D. Autoimmune hemolytic anemia (Correct Answer)
Explanation: ### Explanation **Correct Option: D. Autoimmune Hemolytic Anemia (AIHA)** The key to this question lies in the **age of presentation** and the **transfusion history**. 1. **Age:** A 21-year-old presenting with severe anemia (Hb 5 gm/dL) for the first time suggests an acquired or late-onset condition rather than a severe congenital hemoglobinopathy. 2. **Transfusion History:** The patient has received only **one transfusion** in 21 years despite a very low hemoglobin. This "transfusion-sparing" clinical course rules out Thalassemia Major. 3. **Clinical Features:** Hepatosplenomegaly is common in AIHA due to extramedullary hematopoiesis and splenic sequestration of antibody-coated RBCs. --- ### Why the other options are incorrect: * **A. Thalassemia Major:** This typically presents in **infancy (6–9 months)** as fetal hemoglobin (HbF) levels drop. Without regular monthly transfusions, these patients do not survive to age 21 with a hemoglobin of 5 gm/dL. * **B. Thalassemia Minor:** This is usually an asymptomatic carrier state. While mild anemia may be present, the hemoglobin rarely drops to 5 gm/dL, and hepatosplenomegaly is typically absent. * **C. Thalassemia Intermedia:** While these patients present later than Thalassemia Major and are "transfusion-independent," they usually maintain a hemoglobin between 7–10 gm/dL. A drop to 5 gm/dL at age 21 without a prior history of regular transfusions is more characteristic of an acute hemolytic process like AIHA. --- ### NEET-PG High-Yield Pearls: * **Thalassemia Major:** "Transfusion-dependent"; presents with "Chipmunk facies" and "Hair-on-end" appearance on X-ray. * **AIHA Diagnosis:** The gold standard investigation is the **Direct Coombs Test (Direct Antiglobulin Test)**. * **Clinical Clue:** If a young adult presents with sudden severe anemia and splenomegaly, always consider AIHA or a late-presenting Hereditary Spherocytosis.
Question 10: What is the recommended treatment for Kostmann's syndrome?
- A. Anti-thymocyte globulin plus cyclosporin
- B. Anti-thymocyte globulin plus cyclosporin plus GM-CSF
- C. G-CSF (Correct Answer)
- D. GM-CSF
Explanation: **Explanation:** **Kostmann’s Syndrome** (Severe Congenital Neutropenia) is an autosomal recessive disorder characterized by a maturation arrest of neutrophil precursors in the bone marrow at the **promyelocyte/myelocyte stage**. This leads to absolute neutrophil counts (ANC) frequently below 200/mm³, predisposing infants to life-threatening pyogenic infections. **Why G-CSF is the Correct Answer:** The primary goal of treatment is to increase the production and maturation of neutrophils. **Granulocyte Colony-Stimulating Factor (G-CSF)** is the gold-standard treatment. It effectively increases the ANC in over 90% of patients, significantly reducing the frequency of infections and improving survival. While Hematopoietic Stem Cell Transplant (HSCT) is the definitive cure, G-CSF is the first-line medical management. **Why Other Options are Incorrect:** * **Options A & B:** Anti-thymocyte globulin (ATG) and Cyclosporin are immunosuppressive therapies used for **Aplastic Anemia**, where the pathology is T-cell mediated destruction of stem cells. Kostmann’s is a genetic maturation defect, not an autoimmune process. * **Option D:** **GM-CSF** (Granulocyte-Macrophage CSF) is less effective than G-CSF and is associated with more systemic side effects (like fever and bone pain) without providing a superior neutrophil response in these patients. **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** Most common mutation in Severe Congenital Neutropenia is **ELANE** (autosomal dominant), but the classic **Kostmann’s** specifically refers to the **HAX1** mutation (autosomal recessive). * **Bone Marrow Finding:** Characterized by "maturation arrest" at the promyelocyte stage. * **Malignancy Risk:** Patients have a significantly increased risk of developing **Acute Myeloid Leukemia (AML)** or Myelodysplastic Syndrome (MDS), even with G-CSF treatment. * **Definitive Treatment:** For patients refractory to G-CSF or those developing MDS/AML, **Stem Cell Transplant** is the only curative option.
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